Angora hair nevus syndrome

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Classification according to ICD-10
Q82.5 Congenital non-neoplastic nevus
ICD-10 online (WHO version 2019)

The angora hair nevus syndrome is a very rare special form of the epidermal nevus syndrome .

Synonyms are: angora hair nevus syndrome; Shudder syndrome

The name refers to the first author of the first description from the year 2000 by the Göttingen dermatologist Silvia Schauder and colleagues.

distribution

The frequency is given as less than 1 in 1,000,000, so far two victims have been reported, the cause is not yet known.

literature

  • M. d. Boente, R. Asial, NB Primc, R. Happle: Angora hair nevus. A further case of an unusual epidermal nevus representing a hallmark of angora hair nevus syndrome. In: Journal of dermatological case reports. Vol. 7, No. 2, June 2013, pp. 49-51, doi: 10.3315 / jdcr.2013.1138 , PMID 23858341 , PMC 3710678 (free full text).
  • R. Happle: The group of epidermal nevus syndromes Part I. Well defined phenotypes. In: Journal of the American Academy of Dermatology. Vol. 63, No. 1, July 2010, pp. 1-22, doi: 10.1016 / j.jaad.2010.01.017 , PMID 20542174 (review).

Individual evidence

  1. ^ A b Angora hair nevus syndrome. In: Orphanet (Rare Disease Database).
  2. ↑ S. Schauder, F. Hanefeld, UM Noske, B. Zoll: Depigmented hypertrichosis following Blaschko's lines associated with cerebral and ocular malformations: a new neurocutaneous, autosomal lethal gene syndrome from the group of epidermal naevus syndromes? In: The British journal of dermatology. Vol. 142, No. 6, June 2000, pp. 1204-1207, PMID 10848748 .